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Olivia Xerri, Sawsen Salah, Dominique Monnet, Antoine P Brezin; Acute Posterior Multifocal Pigment Epitheliopathy : visual outcome in untreated patients. Invest. Ophthalmol. Vis. Sci. 2016;57(12):1887.
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© 2017 Association for Research in Vision and Ophthalmology.
Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) is a rare inflammatory eye disease that affects the Retinal Pigment Epithelium and outer retina.The purpose of this study was to describe the visual prognosis and the ocular and extra-ocular features in a cohort of untreated patients
Patients were selected from the department of Ophthalmology at Cochin University Hospital, Paris. Patients were included if they had the typical findings of APMPPE: with whitish or yellowish bilateral placoid lesions and a typical pattern of early hypofluorescence and late hyperfluorescence on fluorescein angiography with an acute onset of the manifestations. Only untreated patients who had been followed for at least 1 month in our department were included. Follow-up examinations included Best Corrected Visual Acuity (BCVA), fundus examination and OCT. Extra-ocular features were also studied.
From 22 patients with a diagnosis of APMPPE, 12 were excluded (7 for lack of follow-up, 3 for atypical features, and 2 who were being treated). Ten patients (9 women, 1 man) fulfilled the study’s criteria with a diagnosis of typical untreated APMPPE. The mean age of patients was 24.5 ± 4.2 years and the mean follow-up was 10.9 ± 13.5 months (median 5.5 months). A macular lesion was observed in 18/20 eyes. More than 5 lesions were observed in 13 eyes. The size of the lesions at presentation ranged from 50 to 250μm in 2/12 eyes, from 250 to 500μm in 11/12 eyes and was greater than 500μm in 10/12 eyes. Lesions were located at the posterior pole in 19/20 eyes and in the mid periphery in 12/20 eyes. Sub-retinal fluid was seen at presentation in 3/15 eyes. Initial mean BCVA was 0.56 ± 0.81 LogMAR [-0.10 to 2.30]. In 9/10 cases, the time interval between manifestations in the first affected eye and the fellow eye was less than 3 days; in one case bilateralisation was observed after 15 days. After 1 month, BCVA had improved to 0.05 ± 0,.089 LogMAR [0 - 0.3] with a decimal BCVA ≥ 0.8 in 17/20 eyes.Prodromal symptoms (headaches and fever in 2 cases, fever in 3 cases and erythema nodosum in 2 cases) were reported in 7/10 patients. A lumbar puncture was performed in 2 cases, with a normal Cerebro Spinal Fluid in 1 case and 1 case of lymphocytic meningitis (121 cells/mm3).
In all of these 10 cases of untreated APMPPE, a favorable natural history was observed.
This is an abstract that was submitted for the 2016 ARVO Annual Meeting, held in Seattle, Wash., May 1-5, 2016.
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